- It is a common ==multisystemic== ==inflammatory== disease that begins more frequently between 20 and 60 years of age and represents the most frequent of all interstitial lung diseases.
- The lungs are almost constantly affected (about 90% of cases) with bilateral pulmonary hilar lymph node involvement and possible simultaneous involvement of the lungs.
- Histopathology features include noncaseating epithelioid granulomas with tightly packed epithelioid cells, Langhans giant cells, and T lymphocytes.
[!INFO] Löfgren’s syndrome:
Lofgren's syndrome is an acute form of the disease characterised by:
[!TIP] Summary
Symmetrical Bilateral Hilar lymphadenopathy (BHL) is a characteristic feature of sarcoidosis and is usually asymptomatic.
#sarcoidosis
- It is a common ==multisystemic== ==inflammatory== disease that begins more frequently between 20 and 60 years of age and represents the most frequent of all interstitial lung diseases.
- Commoner in young adults.
- The lungs are almost constantly affected (about 90% of cases) with bilateral pulmonary hilar lymph node involvement and possible simultaneous involvement of the lungs.
- Histopathology features include noncaseating epithelioid granulomas with tightly packed epithelioid cells, Langhans giant cells, and T lymphocytes.
- These findings are localized in interstitium adjacent to bronchioles as well as around and within vessel walls, pleura, and connective tissue septa. There are Schaumann bodies (laminated concretions of calcium and protein) and asteroid elements (stellate inclusions within giant cells).
[!INFO] Lupus pernio
Lupus pernio = cutaneous sarcoidosis
Lupus vulgaris = cutaneous tuberculosis
Heart can also be involved: Mild to life threatening spectrum.
Conduction: Heart block and arrhythmias
Muscle: Heart failure
Valves: Valvular dysfunction
simulated infarction
pericardial disease
Renal involvement is uncommon but hypercalcemia and hypercalciuria and cause nephrocalcinosis and cause renal failure. Can also cause granulomatous tubulointerstitial nephritis.
Hypopituitarism
Hypoadrenalism
Cranial nerves palsies
There is also elevated angiotensin converting enzyme levels (which is produced by the granulomas).
Hypercalcemia, hypercalciuria and elevated alkaline phosphatase.
[!INFO] Löfgren’s syndrome:
Lofgren's syndrome is an acute form of the sarcoidosis characterised by
- bilateral hilar lymphadenopathy,
- erythema nodosum,
- arthralgia and
- fever
Carries an excellent prognosis
[!INFO] Heerfordt's syndrome
Heerfordt's syndrome
A rare subacute form of [[Sarcoidosis]]
(uveoparotid fever) there is parotid enlargement, fever and uveitis secondary to sarcoidosis
FBC: Leukopenia and eosinophilia + thrombocytopenia (rare) and Mild NCNC anaemia.
CXR: Bilateral Hilar lymphadenopahty - very common!
Hypercalcemia secondary to increased vitamin D production (macrophages activate a vitamin D precursor)
Renal involvement with elevated creatinine is uncommon
[!TIP] Bilateral Hilar lymphadenopathy SCaLP
Causes of bilateral Hilar lymphadenopathy
- Lymphoma,
- pulmonary TB,
- carcinoma of the bronchus,
- sarcoidosis
#hilar-Lymphadenopathy #mediastinal-Lymphadenopathy
Clinical context: "non superficial lymph nodes".
(In contrast, enlarged intraabdominal or retroperitoneal nodes are usually malignant.)
Mediastinal lymphadenopathy is caused by primary lung diseases or systemic diseases with predilection for the mediastinal nodes.
Causes:
From Harrison's:
Young people :
Older people:
A more comprehensive list from the internet book of critical care:
Source
| Infection | Malignancy | Interstitial lung disease | Chronic medication |
|---|---|---|---|
| Tuberculosis (Usually asymmetric) | Lymphoma | Sarcoidosis (usually symmetric) | Phenytoin |
| Non tuberculous mycobacteria | Castleman disease | Berylliosis | Methotrexate |
| Fungi more frequent(histoplasmosis, Coccidioidomycosis) | Kaposi sarcoma | Silicosis | Allopurinol |
| Fungi (less frequentt(Blastomycosis, cryptococcosis) | CLL | Coal workers pneumoconisis | Aspirin |
| Atypical bacteria: | Intrathoracic primary cancer | Amyloidosis | Erythromycin / sulfonamides / Penicillin |
| - Tularemia. - Yersinia pestis (plague). - Anthrax. - Psittacosis. - Coxiella burnetii (Q fever). - Mycoplasma pneumoniae. |
Mets from extrathoracic sites | Mild lymphadenopathy (e.g., 1-1.5 cm) may be seen with various idiopathic interstitial lung diseases, including: - Idiopathic pulmonary fibrosis. - Connective tissue-related interstitial lung disease. - Hypersensitivity pneumonitis. - Organizing pneumonia. - Chronic eosinophilic pneumonia. |
|
| Viral | |||
| - EBV (Epstein Barr virus). - VZV (varicella zoster virus). - Influenza H1N1. |
Granulomatous diseases can cause calcification of lymph nodes.
Calcification is also seen in
[!TIP] Summary
Symmetrical Bilateral Hilar lymphadenopathy (BHL) is a characteristic feature of sarcoidosis and is usually asymptomatic.
#sarcoidosis
- It is a common ==multisystemic== ==inflammatory== disease that begins more frequently between 20 and 60 years of age and represents the most frequent of all interstitial lung diseases.
- Commoner in young adults.
- The lungs are almost constantly affected (about 90% of cases) with bilateral pulmonary hilar lymph node involvement and possible simultaneous involvement of the lungs.
- Histopathology features include noncaseating epithelioid granulomas with tightly packed epithelioid cells, Langhans giant cells, and T lymphocytes.
- These findings are localized in interstitium adjacent to bronchioles as well as around and within vessel walls, pleura, and connective tissue septa. There are Schaumann bodies (laminated concretions of calcium and protein) and asteroid elements (stellate inclusions within giant cells).
[!INFO] Lupus pernio
Lupus pernio = cutaneous sarcoidosis
Lupus vulgaris = cutaneous tuberculosis
Heart can also be involved: Mild to life threatening spectrum.
Conduction: Heart block and arrhythmias
Muscle: Heart failure
Valves: Valvular dysfunction
simulated infarction
pericardial disease
Renal involvement is uncommon but hypercalcemia and hypercalciuria and cause nephrocalcinosis and cause renal failure. Can also cause granulomatous tubulointerstitial nephritis.
Hypopituitarism
Hypoadrenalism
Cranial nerves palsies
There is also elevated angiotensin converting enzyme levels (which is produced by the granulomas).
Hypercalcemia, hypercalciuria and elevated alkaline phosphatase.
[!INFO] Löfgren’s syndrome:
Lofgren's syndrome is an acute form of the sarcoidosis characterised by
- bilateral hilar lymphadenopathy,
- erythema nodosum,
- arthralgia and
- fever
Carries an excellent prognosis
[!INFO] Heerfordt's syndrome
Heerfordt's syndrome
A rare subacute form of [[Sarcoidosis]]
(uveoparotid fever) there is parotid enlargement, fever and uveitis secondary to sarcoidosis
FBC: Leukopenia and eosinophilia + thrombocytopenia (rare) and Mild NCNC anaemia.
CXR: Bilateral Hilar lymphadenopahty - very common!
Hypercalcemia secondary to increased vitamin D production (macrophages activate a vitamin D precursor)
Renal involvement with elevated creatinine is uncommon
[!TIP] Bilateral Hilar lymphadenopathy SCaLP
Causes of bilateral Hilar lymphadenopathy
- Lymphoma,
- pulmonary TB,
- carcinoma of the bronchus,
- sarcoidosis
#hilar-Lymphadenopathy #mediastinal-Lymphadenopathy
Clinical context: "non superficial lymph nodes".
(In contrast, enlarged intraabdominal or retroperitoneal nodes are usually malignant.)
Mediastinal lymphadenopathy is caused by primary lung diseases or systemic diseases with predilection for the mediastinal nodes.
Causes:
From Harrison's:
Young people :
Older people:
A more comprehensive list from the internet book of critical care:
Source
| Infection | Malignancy | Interstitial lung disease | Chronic medication |
|---|---|---|---|
| Tuberculosis (Usually asymmetric) | Lymphoma | Sarcoidosis (usually symmetric) | Phenytoin |
| Non tuberculous mycobacteria | Castleman disease | Berylliosis | Methotrexate |
| Fungi more frequent(histoplasmosis, Coccidioidomycosis) | Kaposi sarcoma | Silicosis | Allopurinol |
| Fungi (less frequentt(Blastomycosis, cryptococcosis) | CLL | Coal workers pneumoconisis | Aspirin |
| Atypical bacteria: | Intrathoracic primary cancer | Amyloidosis | Erythromycin / sulfonamides / Penicillin |
| - Tularemia. - Yersinia pestis (plague). - Anthrax. - Psittacosis. - Coxiella burnetii (Q fever). - Mycoplasma pneumoniae. |
Mets from extrathoracic sites | Mild lymphadenopathy (e.g., 1-1.5 cm) may be seen with various idiopathic interstitial lung diseases, including: - Idiopathic pulmonary fibrosis. - Connective tissue-related interstitial lung disease. - Hypersensitivity pneumonitis. - Organizing pneumonia. - Chronic eosinophilic pneumonia. |
|
| Viral | |||
| - EBV (Epstein Barr virus). - VZV (varicella zoster virus). - Influenza H1N1. |
Granulomatous diseases can cause calcification of lymph nodes.
Calcification is also seen in
" MesPGN may occur in several renal diseases such as IgA nephropathy (commonly), IgM nephropathy, lupus nephritis, and C1q nephropathy". Source
(A) Pathologic lesion (oval shaded area) in the dorsal pontine tegmentum involves both ipsilateral PPRF (or the abducens nucleus) and the ipsilateral MLF; (B) Position of eyeballs, with rightward gaze, the left eye has the impaired adduction, while the abduction of the right eye is intact, except for the nystagmus, and with left gaze, both eyes have impaired horizontal movement (abduction of the left eye and adduction of right eye).